Perifolliculitis capitis abscedens et suffodiens

ARTICLE

A 25-year-old woman has been suffering for 3 years from painful, multiple, bald and fluctuant nodular lesions of the occipital scalp and retroauricular areas, some of which spontaneously disappeared (Fig. 1). In the past year, similar fistulized and ulcerated lesions in the left laterocervical area (Fig. 2) and hidradenitis of the left axillary area have also occurred. The clinico-pathological history of the patient was negative; she had no diabetes, acne or any underlying cutaneous or systemic illness and she wasn't taking any medication or oral contraceptives. The cultures for fungi, aerobic and anaerobic bacteria and alcohol-acid-fast bacilli were negative. A lesional skin biopsy showed a perifollicular inflammatory infiltrate of lymphocytes, plasmacells, eosinophils and neutrophils.

Perifolliculitis capitis abscedens et suffodiens

Dissecting cellulitis of the scalp (DCS), otherwise known as perifolliculitis capitis abscedens et suffodiens, is a rare, chronic suppurative disease mostly affecting Afro-Carribean men in the age range 20-40 years. It was first described by Spitzer in 1903 [1] and named by Hoffmann in 1908 [2]. It is characterized by pustules, nodules, interconnecting abscesses and sinuses that leave atrophic, hypertrophic or keloidal scars. The vertex and occipit of the scalp are the most frequent localization, but perineal and pubic sites are occasionally involved. Although the aetiology is unknown, the frequent association with acne conglobata and hidradenitis suppurativa suggests a common pathogenetic mechanism of follicular retention [3]. A number of therapeutical approaches for DCS have been reported in the literature. Topical, intralesional and systemic steroids [4] and topical and systemic antibiotics with periodic drainage of fluctuant swellings [5] have been used with various degrees of success. Other therapies have also been described, such as isotretinoin [6, 7] alone or in combination with steroids [8], and zinc sulfate [9]. Isotretinoin is the most common treatment even if recurrences after the end of the therapy have frequently been reported [10].

Comments

DSC usually affects the vertex and scalp occipit. In our patient, the lesions were localized to the occipit of the scalp and also to the laterocervical and retroauricular areas, atypical and rare sites, similar to those affected in scrofuloderma. In our case, cultures for fungi, aerobic and anaerobic bacteria and alcohol-acid-fast bacilli were negative. Although isotretinoin is the most frequently used treatment in this disease, we preferred to avoid its administration in our young female patient. So we chose a combination therapy with doxycycline and prednisone to increase the antiinflammatory action of both compounds and to be able to continue treatment for a long time without significant side-effects. We started with doxycycline 200 mg/d and prednisone 50 mg/d. This therapy was progressively reduced every two weeks to a dosage of doxycycline 50 mg/d and prednisone 5 mg/d. After a few weeks of therapy, a clinical improvement was evident and after three months complete healing was achieved. Treatment was discontinued after four months (Fig. 3), and at a two year follow-up there is no evidence of recurrence and the patient shows only scars.

References

1. Spitzer L. Dermatitis follicularis et perifolliculitis conglobata. Dermatol Z 1903; 10: 109-20.

2. Hoffmann E. Perifolliculitis capitis abscedens et suffodiens: case presentation. Dermatol Z 1908; 15: 122-3

3. Lever WF, Schaumburg-Lever G. Follicular occlusion triad (hidradenitis suppurativa, acne conglobata, perifolliculitis capitis abscedens et suffodiens). Arch Dermatol 1992; 128: 1115-7.

4. Adrian RM, Arndt KA. Perifolliculitis capitis: successful control with alternate-day corticosteroids. Ann Plast Surg 1980; 4: 166-9.

5. Ramesh V. Dissecting cellulitis of the scalp in two girls. Dermatologica 1990; 180: 48-50.

6. Dubost-Brama A, Delaporte E, Alfandari S, Piette F, Bergoend H. Folliculite dissequante du cuir chevelu: efficacité de l'isotretinoine. Ann Dermatol Venereol 1994; 121: 328-30.

7. Scerri L, Williams HC, Allen BR. Dissecting cellulitis of the scalp: response to isotretinoin. Br J Dermatol 1996; 134: 1105-8.

8. Shaffer N, Billick RC, Srolovitz H. Perifolliculitis capitis abscedens et suffodiens. Resolution with combination therapy. Arch Dermatol 1992; 128: 1329-31.

9. Berne B, Venge P, Ohman S. Perifolliculitis capitis abscedens et suffodiens (Hoffman). Complete healing associated with oral zinc therapy. Arch Dermatol 1985; 121: 1028-30.

10. Schewach-Millet M, Ziv R, Shapira D. Perifolliculitis capitis abscedens et suffodiens treated with isotretinoin (13 cis-retinoic acid). J Am Acad Dermatol 1986; 15: 1291-2.